A 46-year-old man presented at the outpatient cardiology center with a 5-year history of paroxysmal atrial fibrillation. During this period, he suffered several palpitations triggered by alcohol or fatigue, with cardioversion of atrial fibrillation to sinus rhythm through intravenous administration of amiodarone. This time he presented with palpitations lasting for 3 days, without chest pain, shortness of breath, cyanosis, or edema. An electrocardiogram revealed atrial fibrillation. A transthoracic echocardiogram showed left atrial enlargement (48 mm), near the upper limit of the left ventricle (54 mm), right atrial enlargement (41 mm), left ventricular ejection fraction of 57%, as well as mild-to-moderate mitral regurgitation, mild aortic regurgitation, and mild tricuspid regurgitation. Unusually, the transthoracic echocardiogram revealed a dilated right coronary artery with an internal diameter of approximately 8 mm, originating from the ascending aorta (Figure 1A). An abnormal blood flow directed towards the pulmonary artery (Figure 1B) could indicate an anomalous connection between the pulmonary artery and the left coronary artery. An abnormal vascular structure with reversed flow detectable inside the interventricular septum on color Doppler was observed (Figure 1C), which suggested the reversed flow from the right coronary artery to the left coronary artery through coronary collateral vessels.
Based on these image characteristics, the primary diagnosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) was proposed. Furthermore, this diagnosis was confirmed by coronary computed tomography angiography (Figure 1D–E) and coronary angiography (Figure 1F), which both revealed a left coronary artery anomaly originating from the pulmonary artery.
The patient then underwent surgery by ligation of the abnormal opening of the left coronary artery and coronary artery bypass grafting to establish circulation from the aorta to the left anterior descending branch. Normal myocardial perfusion was restored after surgery. One month after surgery, the patient was asymptomatic. Physical examination showed no signs of heart failure. An electrocardiogram revealed atrial fibrillation. The transthoracic echocardiogram showed a residual 2 mm shunt bundle at the opening from the left coronary artery to the pulmonary artery.
ALCAPA, also known as Bland–White–Garland syndrome, is considered a rare congenital anomaly of coronary arteries, which occurs in 1 out of 300 000 live births. Without treatment, 90% of infants die within the first year of life. Survival to adulthood for patients with ALCAPA syndrome is extremely rare [1]. We reported the case of a patient who survived 46 years without obvious left ventricular dysfunction. This is attributed to the collateral circulation between the left and right coronary arteries which ensures adequate blood supply to the heart. Despite this, chronic left ventricular subendocardial ischemia may occur, posing a high risk of sudden cardiac death due to malignant ventricular arrhythmias. Transthoracic echocardiogram provides great value as the first-choice method for the correct diagnosis of ALCAPA. Characteristic findings, such as a dilated right coronary artery, abnormal blood flow towards the pulmonary artery, and abnormal vascular structure with reversed flow inside the interventricular septum, are key diagnostic indicators of ALCAPA. Early diagnosis and prompt surgical intervention are crucial for improving outcomes and preventing complications such as left ventricular dysfunction and sudden cardiac death [2].
Article information
Conflict of interest: None declared.
Funding: This work was funded by the Innovation Team and Talents Cultivation Program of the National Administration of Traditional Chinese Medicine (No. ZYYCXTD-C-202203).
Open access: This article is available in open access under Creative Common Attribution-Non-Commercial-No Derivatives 4.0 International (CC BY-NC-ND 4.0) license, which allows downloading and sharing articles with others as long as they credit the authors and the publisher, but without permission to change them in any way or use them commercially. For commercial use, please contact the journal office at polishheartjournal@ptkardio.pl
